Disease pattern that belongs to the cystic nephropathies. Renal dysplasia develops as a result of abnormal differentiation of the metanephritic tissue and the resulting abnormal development of the kidney (Hofmann 2005). Hydronephrosis begins to form as early as intrauterine (Manski 2019). In renal dysplasia with cysts, there is a restricted functional parenchyma, which can occur unilaterally or bilaterally. Smaller cysts are often found.
Kidney dysplasias are rather rare. They do not follow a clear pattern of inheritance; sometimes there is at most a familial clustering. I do not have more detailed figures on this (Herold 2021).
Renal dysplasia develops as a result of abnormal differentiation of the metanephritic tissue and consequent abnormal development of the kidney (Hofmann 2005). Hydronephrosis already begins to form intrauterine (Manski 2019).
Bilateral infestation can lead to chronic renal failure (Gimpel 2019). Unilateral infestations are usually asymptomatic (Ganten 2013).
The diagnosis of renal dysplasia can be confirmed by the typical histology (see d.) (Manski 2019)
In the case of unilateral infestation, there are usually no abnormalities.
Primitive tubules lined with epithelium are found here. The tubules are surrounded by smooth muscle cells and concentric rings of connective tissue and smooth muscle cells. It is not uncommon to find additional cartilage (Manski 2019).
Multicystic dysplastic kidneys (Gimpel 2019).
Treatment of possible renal insufficiency in case of bilateral infestation (see d.).
In case of unilateral involvement, the prognosis is good. In case of bilateral involvement, chronic renal insufficiency determines the further course.
Gimpel C et al. (2019) Imaging diagnosis in children with renal cysts and cystic kidneys. Monthly Journal of Pediatrics (167) 530 - 538.